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Systemic sclerosis (SSc), a chronic multisystem autoimmune disease characterized by fibrosis of the skin and internal organs and vasculopathy, has a high burden of mortality. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH), which affects up to 10% of individuals and results in up to 15 years of life loss. Best practice recommendations are for asymptomatic patients with SSc and SSc-spectrum disorder to be screened annually for the early detection of SSc-PAH. Recently published data from large registries have shown improvements in the long-term outcomes in patients who are diagnosed with SSc-PAH because of systematic annual screening. This review will address the current clinical and research implications of the screening for the early detection of SSc-PAH.
Cite this article as: Brown ZR, Nikpour M. Screening for pulmonary arterial hypertension in systemic sclerosis: Now or never! Eur J Rheumatol 2020; 7(Suppl 3): S187-92.